BST1

Chr 4

bone marrow stromal cell antigen 1

Also known as: CD157, cADPR2

BST1 encodes bone marrow stromal cell antigen-1, which catalyzes the synthesis and hydrolysis of cyclic ADP-ribose from NAD+, serving as a second messenger that regulates intracellular calcium mobilization and facilitates pre-B-cell growth. Mutations cause autosomal recessive early-onset Parkinson disease, representing a rare monogenic form of parkinsonism in children and young adults. The gene shows minimal constraint against loss-of-function variants (pLI near zero), consistent with recessive inheritance where heterozygous carriers are typically unaffected.

Summary from RefSeq, UniProt
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0
Active trials
28
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.36
LOEUF
GOF
Mechanism· predicted
Clinical SummaryBST1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.36LOEUF
pLI 0.000
Z-score 0.38
OE 0.91 (0.621.36)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.10Z-score
OE missense 1.02 (0.901.16)
173 obs / 169.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.91 (0.621.36)
00.351.4
Missense OE1.02 (0.901.16)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 17 / 18.8Missense obs/exp: 173 / 169.2Syn Z: 0.74
DN
0.4785th %ile
GOF
0.7028th %ile
LOF
0.3842th %ile

The highest-scoring mechanism for this gene is gain-of-function.

GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BST1 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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